Transition Intervention for Adolescents With Congenital Heart Disease

Background

There is little evidence regarding the efficacy of interventions to prepare adolescents with congenital heart disease (CHD) to enter adult care.

Slower Skeletal Muscle Oxygenation Kinetics in Adults With Complex Congenital Heart Disease

BackgroundAdults with complex congenital heart disease (CHD) show reduced aerobic exercise capacity and impaired skeletal muscle function compared with healthy peers. Peripheral muscle factors are presumed to be important contributors to the aerobic capacity, but the mechanisms are poorly understood. The aim of the present study was to investigate differences between adults with CHD and controls in muscle oxygenation kinetics at rest, and during and after exercise.MethodsSeventy-four patients with complex CHD (mean age 35.6 ± 14.3 years, female n = 22) were recruited. Seventy-four age- and sex-matched subjects were recruited as controls. Muscle oxygenation was successfully determined on the anterior portion of the deltoid muscle using near-infrared spectroscopy in 65 patients and 71 controls. Measurements were made at rest, during isotonic shoulder flexions (0-90°) to exhaustion, and during recovery.ResultsThe patients with CHD performed fewer shoulder flexions (40 ± 17 vs 69 ± 40; P < 0.001), had lower muscle oxygen saturation (StO₂) at rest (58 ± 18% vs 69 ± 18%; P < 0.001), slower desaturation rate at exercise onset (?9.7 ± 5.9 vs ?15.1 ± 6.5% StO₂ × 3.5 s^?1, P <0.001), and slower resaturation rate post exercise (4.0 ± 2.7 vs 5.4 ± 3.6% StO₂ × 3.5 s^?1; P = 0.009) compared with the controls.ConclusionsIn comparison with age- and sex-matched controls, adults with complex CHD had slower oxygenation kinetics. This altered skeletal muscle metabolism might contribute to the impaired skeletal muscle endurance capacity shown and thereby also to the reduced aerobic capacity in this population.     

Physical Activity Is Associated With Better Vascular Function in Children and Adolescents With Congenital Heart Disease

BackgroundAortic stiffness is an important marker of cardiovascular risk and is elevated in children and adolescents with congenital heart disease (CHD) compared with healthy children; however, in children with CHD, little is known about the interaction between aortic stiffness and physical activity—a key determinant of aortic stiffness.MethodsFor this cross-sectional cohort study, we recruited children and adolescents aged 9-16 years with moderate-to-complex CHD from British Columbia Children’s Hospital and travelling partnership clinics across the province of British Columbia and the Yukon territory. Mean daily minutes of moderate-to-vigorous physical activity were objectively assessed using an ActiGraph accelerometer worn over the right hip during waking hours for 7 days. Aortic pulse wave velocity (cm/s) was measured using standard 2-dimensional echocardiography and Doppler ultrasound.ResultsParticipants (n = 104, 61% male; 85% consent rate) had a mean (standard deviation) age of 12.4 (2.4) years. Daily moderate-to-vigorous physical activity was 46.7 (20.0) minutes/d, with 25% meeting guidelines of ≥ 60 minutes of moderate-to-vigorous physical activity per day. Mean (standard deviation) aortic pulse wave velocity was 490.5 (161.9) cm/s, which was not significantly different between cardiac diagnoses. Higher levels of moderate-to-vigorous physical activity were associated with lower aortic pulse wave velocity (r = −0.226, P = 0.021).ConclusionIn children and adolescents with CHD, higher levels of physical activity are associated with better vascular function. Given this association, promoting physical activity should be a high priority in the care of children and adolescents with CHD.     

Cardiovascular Risk Factors in Patients With Congenital Heart Disease

Survival of infants born with congenital heart disease (CHD) is improving tremendously and although most of them have mild lesions, others might be considered as only being palliated and undergo many medical or surgical interventions. Patients with CHD will be exposed to the same problematic of the modern lifestyle such as increased prevalence of obesity, decreased physical activities, and exposure to smoking, which leads to acquired cardiovascular disease. We specifically investigated specific cardiovascular risk factors such as: malnutrition, smoking exposure, hypertension, integrity of the coronary and systemic arteries, thromboembolism, ventricular dysfunction, inflammation, and arrhythmias. Patients with CHD are often submitted to extremes of nutrition: as infants, they often do not meet their metabolic requirements, and as they grow older, they tend to exceed them, as seen in the general population. Some heart lesions are more prone to systemic hypertension throughout life, such as coarctation of the aorta, but surprisingly other lesions are also prone to hypertension such as Ebstein anomaly, pulmonary valve stenosis, or regurgitation. Early coronary artery atherosclerosis is also a concern in these patients. Lesions typically at risk are localized in zones of increased turbulence or high pressure or having had previous surgical manipulations. Thromboembolism is also frequent and mostly associated with arrhythmias, heart failure, multiple catheterizations, and specific surgical repairs. Finally, the complexity of heart lesions or abnormal hemodynamics lead to inflammation, heart failure, or arrhythmias. These complex interactions of risk factors ultimately lead to a decreased life expectancy.     

Structural Brain Alterations and Their Associations With Function in Children,Adolescents, and Young Adults With Congenital Heart Disease

Most neonates who receive surgery for complex congenital heart disease (CHD) will survive well into adulthood, however, many of them will face functional challenges at one point during their life as a consequence of their atypical neurodevelopment. Recent advances in neuroscience and the increasing accessibility of magnetic resonance imaging have allowed numerous studies to identify the nature and extent of the brain alterations that are particular to survivors with CHD. Nevertheless, and considering that the range of outcomes is broad in this population, the functional consequences of these brain differences is not always evident. In this review, we summarize the present state of knowledge regarding the structure-function relationships evaluated in children, adolescents, and young adults with CHD using structural magnetic resonance imaging. Overall smaller total and regional brain volume, as well as lower fractional anisotropy in numerous brain regions, were frequently associated with lower cognitive outcomes including executive functioning and memory in adolescents and young adults with CHD. However, we identify several gaps in knowledge including the limited number of prospective investigations involving neonatal imaging and follow-up during childhood or adolescence, as well as the need for studies that evaluate a broader range of functional outcomes and not only the cognitive abilities. Future interdisciplinary investigations using multimodal imaging techniques could help address these gaps.     

右心声学造影诊断分流性先天性心脏病的应用价值

目的:总结右心声学造影在确诊或协助诊断分流性先天性心脏病的应用价值.方法:回顾分析我院2003-03至2009-07常规超声心动图无法确诊而行右心声学造影明确心内有无分流或腔静脉回流是否异常患者276例.根据临床需要和右心声学造影阳性结果分为三组.结果:第一组70例,有临床症状常规超声检查心脏结构正常,右心声学造影检查阳性者34例(48.6％,34/70),阴性者36例(51.4％,36/70).第二组129例,其中右心声学造影阳性发现并提示肺动脉高压原因者84例(65.1％,84/129),阳性发现与肺动脉高压程度不匹配者34例(26.4％,34/129).第三组77例,心脏结构异常但肺动脉压不高,右心声学造影阳性者54例(70.1％).结论:右心声学造影在常规超声检查无法确诊的分流性先天性心脏病、鉴别肺动脉高压原因、了解腔静脉回流路径中具有重要价值,作为常规超声心动图诊断分流性先心病的补充.     

Health Behaviors among Adolescents and Young Adults with Congenital Heart Disease

Objectives. Document the frequency of substance use and oral hygiene among adolescents and young adults with moderate to complex congenital heart disease (CHD). Background. Patients' knowledge of health behaviors, including substance use and oral hygiene, has been examined among patients with CHD, but patients' actual behavior has not been studied. Understanding patients' behavior is needed to inform interventions that enhance healthy lifestyles, and in turn encourage patients to better care for their own health. Methods. Young adults (19 or 20 years old) and older adolescents (16–18 years old) with moderate or complex CHD (n = 328) reported their substance use (i.e., smoking, marijuana, alcohol, other illicit drugs) and oral hygiene. Rates of these health behaviors were contrasted with comparison samples of peers of the same age. Results. Just over half of the young adults (54%) and over one‐quarter of the adolescents (28%) reported significant substance use (i.e., smoking cigarettes on more than 2 days, using marijuana or other illicit drugs at least once, or binge drinking) during the previous 30 days. Rates of significant substance use among the patients with CHD were either comparable to, or lower than, rates in comparison samples of similar aged peers. Only about 15% of the patients with CHD had excellent oral hygiene (i.e., self‐reported annual teeth cleaning by dentist, brushing and flossing daily); however, virtually all patients (>95%) brushed daily. Patients with CHD generally had comparable oral hygiene to comparison samples in previously published data. Conclusions. Many older adolescents and young adults with CHD are engaging in behaviors that may compromise their health. The impact of substance use and poor dental hygiene warrants further investigation. Nevertheless, health behaviors should be routinely discussed in this population.     

Lesion-based Patterns of Morbidity and Mortality in Hospitalized Adolescents with Congenital Heart Disease

Objective: The objective of this analysis is to describe the characteristics and morbidity during hospitalizations among adolescents with congenital heart disease (AdoCHD) from the Pediatric Health Information System (PHIS) database. Methods: The PHIS database was queried for all AdoCHD admissions aged 12–18 years (1/1/2004–12/31/2013). Major forms of CHD were identified by their International Classification of Diseases, ninth revision codes, further verified based on their secondary diagnosis and/or procedure codes. Patient characteristics, diagnoses, procedures and vital status were assessed. Results: In total, there were 4,267 adolescents admitted to 42 Children’s Hospitals, 58.3% were males, 24.6% single ventricle (SV) patients, 64.1% bi-ventricle (BV), and 11.3% could not be classified. They accounted for 8,512 hospitalizations (41,240 total hospital days), of which 31.6% were intensive care unit (ICU) stays. ICU stay was similar for the SV and BV patients with similar duration of mechanical ventilation between the two groups. Overall, the most common CHD among in-patients was tetralogy of Fallot (TOF, 36.4%). Larger proportion of the BV AdoCHD admissions were for elective surgical and electrophysiological procedures. There were 109 (2.5%) heart transplantations (1.3% SV vs. 0.6% BV) and 120 in-hospital deaths (2.8%) (1.1% SV vs. 1.3% BV). Hypoplastic left heart syndrome was the most common diagnosis in transplanted patients (46%) and those who died (28%); TOF (29%) was frequent in 91 (2.1%) patients who had cardiac arrests. Conclusions: Different hospitalization patterns exist for BV and SV AdoCHD. Recognizing this risk may encourage directing resources toward optimizing long-term care of CHD patients.     

Cardiopulmonary Response to Exercise at High Altitude in Adolescents with Congenital Heart Disease

Objective: To extend our knowledge on tolerance of acute high-altitude exposure and hemodynamic response to exercise in adolescents with congenital heart disease (AscCHD) without meaningful clinical or functional restriction. Methods: A symptom limited cardiopulmonary exercise stress test and a non-invasive cardiac output measurement during steady state exercise were performed at 540 m and at 3454 m a.s.l. Symptoms of acute mountain sickness were noted. Results: We recruited 21 healthy controls and 16 AscCHD (59% male, mean age 14.7 ± 1.1 years). Three subjects (2 controls, 1 AscCHD) presented light symptoms of acute mountain sickness (dizziness and headache). During the symptom limited exercise test at lowland, control subjects showed a significantly higher power to weight index (3.5 ± 0.6 W/kg vs. 3.0 ± 0.7 W/kg, p < 0.001), heart rate (188.8 ± 10.4 1/min vs. 179.4 ± 13.1 1/min, p < 0.050) and ventilation (92.8 ± 22.9 l/min vs. 75.4 ± 18.6 l/min, <0.050). At altitude, power to weight index only remained significantly higher in the control group (2.8 ± 0.6 W/kg vs. 2.6 ± 0.6 W/kg, p < 0.001). Pulmonary blood flow (PBF) at lowland showed no difference between the control and the AscCHD group, neither at rest (5.4 ± 0.8 l/min vs. 5.1 ± 0.9 l/min, p = 0.308), nor during the steady state test (10.6 ± 2.4 l/min vs. 10.5 ± 2.0 l/min, p = 0.825). At high altitude, PBF increased by 110% and 112%, respectively (12.8 ± 2.32 l/min vs. 12.5 ± 3.0 l/min; intergroup difference: p = 0.986). Conclusions: High altitude exposure was well tolerated in an unselected group of AscCHD. No significant difference in the cardio-pulmonary adaptation to a control group was noted during a steady state exercise. Symptoms of minor acute mountain sickness did occur, which should however not be misinterpreted as signs of hemodynamic maladaptation.